Muscular Dystrophy
Muscular Dystrophy - Related Videos
05:54
A Simple and Low-cost Assay for Measuring Ambulation in Mouse Models of Muscular Dystrophy
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Cited by 10 •
2017
This protocol describes a flexible, low-cost system for measuring mouse ambulation in an open field activity assay. We show that a 6-minute ambulation assay based on this system detects a decrease in voluntary movement in mdx mice, and accurately distinguishes improvement in a muscle-specific rescue of these animals.
10:30
Multi-exon Skipping Using Cocktail Antisense Oligonucleotides in the Canine X-linked Muscular Dystrophy
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Cited by 23 •
2016
Exon skipping is currently a most promising therapeutic option for Duchenne muscular dystrophy (DMD). To expand the applicability for DMD patients and to optimize the stability/function of the resulting truncated dystrophin proteins, a multi-exon skipping approach using cocktail antisense oligonucleotides was developed and we demonstrated systemic dystrophin rescue in a dog model.
01:21
Satellite Stem Cells and Muscular Dystrophy
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2023
Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
14:10
Isometric and Eccentric Force Generation Assessment of Skeletal Muscles Isolated from Murine Models of Muscular Dystrophies
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Cited by 85 •
2013
Muscle function measurements contribute to the evaluation of potential therapeutics for muscle pathology, as well as to the determination of mechanisms underlying physiology of this tissue. We will demonstrate the preparation of the extensor digitorum longus and diaphragm muscles for functional testing. Protocols for isometric and eccentric contractions will be shown, as well as differences in results between dystrophic muscles, representing a pathological state, and wildtype muscles.
09:39
Modeling Myotonic Dystrophy 1 in C2C12 Myoblast Cells
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Cited by 7 •
2016
In this protocol, we present the procedures in establishing myotonic dystrophy 1 myoblast models, including optimized C2C12 cell maintenance, gene transfection/transduction, and myocyte differentiation.
FAQs
Genetic mutations can disrupt proteins that help maintain skeletal muscle fiber structure and support repair. As a result, muscle fibers experience repeated injury without effective restoration. Over time, damaged tissue degenerates and may be replaced by fat or connective tissue, which contributes to progressive changes in strength, movement, independence, and everyday functioning.
When functional muscle tissue is progressively replaced by fat or connective tissue, the muscles have less capacity to support movement. This physical change can make previously manageable activities more difficult and increase reliance on other people. The resulting changes are psychologically important because they may affect autonomy, self-perception, participation in daily life, and quality of life.
Progressive changes in physical ability can require a person to adapt expectations, routines, and views of independence. These adjustments may influence emotional wellbeing and identity as the individual responds to altered movement and increasing dependence on caregivers. Psychological support focuses on coping with these changes while recognizing the person’s continuing needs, relationships, and participation in care.
Family relationships can become especially important when changing abilities increase dependence on caregivers. The condition may affect how responsibilities, support, and communication are managed within the family. Considering these relationships during psychological care can help address coping and emotional wellbeing while supporting participation in treatment and daily decisions.
Psychological assessment considers how chronic disability and changing physical abilities relate to emotional wellbeing, identity, coping, and family relationships. It can also explore how dependence on caregivers affects participation in care and everyday life. This information helps identify supportive needs alongside physical and rehabilitative treatment rather than focusing only on muscle-related changes.
Supportive interventions can promote adaptation to changing abilities, strengthen participation in care, and address emotional wellbeing. Their focus may include coping with chronic disability, maintaining a coherent sense of identity, and navigating caregiver dependence or family concerns. Used alongside physical and rehabilitative treatment, psychological support can contribute to improved quality of life.
Psychology adds a patient-centered perspective to care by examining how physical changes affect thoughts, emotions, relationships, and behavior. This perspective complements physical and rehabilitative treatment, helping care teams recognize concerns that may influence participation and adaptation. It also connects clinical support with broader outcomes, including independence, family functioning, and quality of life.